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cardiomyopathy pediatric

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https://www.readbyqxmd.com/read/28220574/selective-use-of-the-electrocardiogram-in-pediatric-preparticipation-athletic-examinations-among-pediatric-primary-care-providers
#1
Bradley C Clark, Joshua M Hayman, Charles I Berul, Kristin M Burns, Jonathan R Kaltman
OBJECTIVES: Recent literature examining insurance administrative data suggests that a selective approach, with concurrent history and physical exam (H&P), for obtaining an electrocardiogram (ECG) as a part of a preparticipation examination (PPE) for pediatric athletes is commonly used in the primary care setting demonstrating a high rate of disease detection. We sought to understand practice patterns of providers with regard to usage of ECG as a part of PPE. METHODS: Utilizing an online survey, we queried primary care providers regarding their practice patterns, rationale, and concerns regarding use of ECGs as a part of the PPE...
February 21, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28214152/clinical-and-genetic-diagnosis-of-familial-hypertrophic-cardiomyopathy-results-in-pediatric-cardiology
#2
Bárbara Cardoso, Inês Gomes, Petra Loureiro, Conceição Trigo, Fátima Ferreira Pinto
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is most often of autosomal dominant inheritance with incomplete penetrance and variable expression. The main purpose of family screening is to identify relatives with unrecognized HCM and to monitor those at risk for disease, in order to minimize complications and to assess risk of sudden cardiac death. The ESC and ACCF/AHA guidelines on the diagnosis and management of HCM recommend the screening of child relatives from the age of 10-12 years...
February 14, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28193717/differences-in-presentation-and-outcomes-between-children-with-familial-dilated-cardiomyopathy-and-children-with-idiopathic-dilated-cardiomyopathy-a-report-from-the-pediatric-cardiomyopathy-registry-study-group
#3
Paolo Rusconi, James D Wilkinson, Lynn A Sleeper, Minmin Lu, Gerald F Cox, Jeffrey A Towbin, Steven D Colan, Steven A Webber, Charles E Canter, Stephanie M Ware, Daphne T Hsu, Wendy K Chung, John L Jefferies, Christina Cordero, Steven E Lipshultz
BACKGROUND: Research comparing the survival of children with familial dilated cardiomyopathy (FDCM) to that of children with idiopathic dilated cardiomyopathy (IDCM) has produced conflicting results. METHODS AND RESULTS: We analyzed data from children with FDCM or IDCM using the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry. Compared to children with IDCM (n=647), children with FDCM (n=223) were older (mean 6.2 versus 4.5 years, P<0...
February 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28193509/pilot-study-analyzing-automated-ecg-screening-of-hypertrophic-cardiomyopathy
#4
Matthew Campbell, Xuefu Zhou, Chia Han, Hedayat Abrishami, Gregory Webster, Christina Y Miyake, Christopher T Sower, Jeffery B Anderson, Timothy K Knilans, Richard J Czosek
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is one of the leading causes of sudden cardiac death in athletes. However, pre-participation ECG screening has often been criticized for failing to meet cost-effectiveness thresholds, in part because of high false-positive rates and the cost of ECG screening itself. OBJECTIVES: To assess the testing characteristics of an automated ECG algorithm designed to screen for HCM in a multi-institutional pediatric cohort. METHODS: ECGs from patients with HCM aged 12 - 20 years from 3 pediatric institutions were screened for ECG criteria for HCM using a previously described automated computer algorithm developed specifically for HCM ECG screening...
February 10, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28183324/identification-of-taz-mutations-in-pediatric-patients-with-cardiomyopathy-by-targeted-next-generation-sequencing-in-a-chinese-cohort
#5
Jian Wang, Ying Guo, Meirong Huang, Zhen Zhang, Junxue Zhu, Tingliang Liu, Lin Shi, Fen Li, Huimin Huang, Lijun Fu
BACKGROUND: Barth syndrome (BTHS) is a rare X-linked recessive disease characterized by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. Early diagnosis and appropriate treatment may improve the prognosis of this disease. The purpose of this study is to determine the role of targeted next-generation sequencing (NGS) in the early diagnosis of BTHS in children with cardiomyopathy. METHODS: During the period between 2012 and 2015, a gene panel-based NGS approach was used to search for potentially disease-causing genetic variants in all patients referred to our institution with a clinical diagnosis of primary cardiomyopathy...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28169967/a-pediatric-case-with-takotsubo-cardiomyopathy-and-neurogenic-pulmonary-edema-due-to-an-epidural-hemorrhage
#6
Yuki Nakamori, Noriko Miyazawa, Kenji Yoshitani, Shinichi Yamamoto
No abstract text is available yet for this article.
February 6, 2017: Journal of Neurosurgical Anesthesiology
https://www.readbyqxmd.com/read/28162930/a-randomized-study-of-autologous-bone-marrow-derived-stem-cells-in-pediatric-cardiomyopathy
#7
E Sian Pincott, Deborah Ridout, Margaret Brocklesby, Angus McEwan, Vivek Muthurangu, Michael Burch
BACKGROUND: Bone marrow mononuclear cell fraction has been used as therapy for dilated cardiomyopathy in adults. Although case series are reported, there are no randomized controlled studies in children. METHODS: We designed a randomized, crossover, controlled pilot study to determine safety and feasibility of intracoronary stem cell therapy in children. The primary safety end-point was freedom from death and transplantation or any complication that could be considered related to bone marrow injection or anesthesia (e...
January 5, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28146440/sudden-collapse-of-a-preschool-aged-child-on-the-playground
#8
RoseAnn L Scheller, Laurie H Johnson, Michelle C Caruso, Angela Lorts
PURPOSE: Primary cardiac events are rare in children. There is little information in the literature regarding the most appropriate emergency department (ED) management of this type of pediatric patient, especially with regard to medication use. SUMMARY: This case highlights the pediatric ED evaluation and treatment of sudden collapse in a child with an ultimate diagnosis of hypertrophic cardiomyopathy. Cardiac disorders represent 2% to 6% of cases of pediatric syncope presenting to EDs, particularly if there are previous prodromal symptoms and/or a history of exertion...
February 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28130338/exosomes-from-pediatric-dilated-cardiomyopathy-patients-modulate-a-pathological-response-in-cardiomyocytes
#9
Xuan Jiang, Juliana Sucharov, Brian L Stauffer, Shelley D Miyamoto, Carmen C Sucharov
Stimulation of the renin-angiotensin-aldosterone system (RAAS) and β-adrenergic receptors plays an important role in adult heart failure (HF). Despite the demonstrated benefits of RAAS inhibition and β-adrenergic receptor blockade in adult HF patients, no substantial improvement in survival rate has been observed in children with HF. This suggests that the underlying disease mechanism is uniquely regulated in pediatric HF. Here, we show that treatment of human induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) and neonatal rat ventricular myocytes (NRVMs) with serum from pediatric dilated cardiomyopathy (DCM) patients induces pathologic changes in gene expression, which occurs independently of the RAAS and adrenergic systems, suggesting that serum circulating factors play an important role in cardiac remodeling...
January 27, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28129792/intramuscular-injection-of-human-umbilical-cord-derived-mesenchymal-stem-cells-improves-cardiac-function-in-dilated-cardiomyopathy-rats
#10
Chenggang Mao, Xu Hou, Benzhen Wang, Jingwei Chi, Yanjie Jiang, Caining Zhang, Zipu Li
BACKGROUND: Stem cells provide a promising candidate for the treatment of the fatal pediatric dilated cardiomyopathy (DCM). This study aimed to investigate the effects of intramuscular injection of human umbilical cord-derived mesenchymal stem cells (hUCMSCs) on the cardiac function of a DCM rat model. METHODS: A DCM model was established by intraperitoneal injections of doxorubicin in Sprague-Dawley rats. hUCMSCs at different concentrations or cultured medium were injected via limb skeletal muscles, with blank medium injected as the control...
January 28, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28114151/factors-determining-the-uptake-of-influenza-vaccination-among-children-with-chronic-conditions
#11
Janita Pak Chun Chau, Suzanne Hoi Shan Lo, Kai Chow Choi, Matthew Hoi Kin Chau, Danny Wah Kun Tong, Tany Kwong, David R Thompson
BACKGROUND: Studies report the influenza vaccination uptake rate among children with chronic conditions is alarmingly low. In Hong Kong, there has been no study examining parental decision making about influenza vaccination for children with chronic conditions, thereby limiting the knowledge base to inform the development of specific strategies to improve influenza vaccination rates. AIM: To identify factors determining the uptake of influenza vaccination among children with chronic conditions...
January 19, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28108756/left-ventricular-noncompaction-cardiomyopathy-in-pediatric-patients-a-case-series-of-a-clinically-heterogeneous-disease
#12
Umang Gupta, Pooja Makhija
Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications, and cardiac arrhythmias on the other (Kim et al in J Am Coll Cardiol 53: 2009, 2009). Further, the progression of the condition is highly variable...
January 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28103933/quantification-of-myocardial-deformation-in-children-by-cardiovascular-magnetic-resonance-feature-tracking-determination-of-reference-values-for-left-ventricular-strain-and-strain-rate
#13
Florian André, Daniëlle Robbers-Visser, Astrid Helling-Bakki, Angela Föll, Andreas Voss, Hugo A Katus, Willem A Helbing, Sebastian J Buss, Joachim G Eichhorn
BACKGROUND: The objective assessment of global and regional cardiac function in children has shown to be clinically relevant but is challenging to conduct. Cardiovascular magnetic resonance (CMR) has emerged as a valuable diagnostic modality especially in patients with cardiomyopathy or congenital heart disease. However, data on the normal cardiac deformation in children assessed by CMR is lacking at present. Thus, the aim of this study was to provide reference values for cardiac strain and strain rate in children and adolescents derived from CMR feature tracking (FT) measurements...
December 5, 2016: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28098235/molecular-characterization-of-pediatric-restrictive-cardiomyopathy-from-integrative-genomics
#14
Tara N Rindler, Robert B Hinton, Nathan Salomonis, Stephanie M Ware
Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28070248/childhood-cardiomyopathies-a-study-in-tertiary-care-hospital-in-upper-egypt
#15
Mohamed Abd Elaal Bakeet, Montaser Mohamed Mohamed, Ahmed Ahmed Allam, Rania Gamal
INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital...
November 2016: Electronic Physician
https://www.readbyqxmd.com/read/28040191/left-ventricular-function-in-children-and-adolescents-with-arrhythmogenic-right-ventricular-cardiomyopathy
#16
Paweena Chungsomprasong, Robert Hamilton, Wietske Luining, Meena Fatah, Shi-Joon Yoo, Lars Grosse-Wortmann
The aim of this study was to determine if left ventricular (LV) contractility is reduced in children with arrhythmogenic right ventricular cardiomyopathy (ARVC). For this retrospective study, children and adolescents undergoing a workup for ARVC were characterized according to the revised Task Force Criteria (rTFC). LV strain, rotation, and torsion were measured by feature-tracking cardiovascular magnetic resonance imaging (CMR). Of 142 pediatric patients, 41% had no, 23% possible, 20% borderline, and 16% definite ARVC...
December 2, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/28039505/factors-supporting-cardiomyopathy-screening-among-at-risk-adult-survivors-of-pediatric-malignancies
#17
Cheryl L Cox, Liang Zhu, Rohit P Ojha, Brenda D Steen, Susan Ogg, Leslie L Robison, Melissa M Hudson
PURPOSE: Anthracyclines and chest irradiation place adult survivors of childhood cancer at risk of cardiomyopathy; many survivors do not obtain the recommended screening. Based on our recent clinical trial, the addition of telephone counseling to a printed survivorship care plan more than doubled survivors' risk-based screening. Here, we sought to measure the impact of specific factors targeted in the intervention for their impact on survivors' screening participation. METHODS: Study population-survivors participating in a randomized longitudinal intervention trial...
April 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28033083/the-hemodynamic-effects-and-safety-of-repetitive-levosimendan-infusions-on-children-with-dilated-cardiomyopathy
#18
Pertti Suominen, Niklas Mattila, Olle Nyblom, Paula Rautiainen, Maila Turanlahti, Otto Rahkonen
BACKGROUND: Limited treatment options are available for children with decompensated dilated cardiomyopathy (DCM), while they wait for either functional recovery or heart transplantation. We evaluated the safety of repetitive levosimendan infusions and short-term and long-term impacts of the therapy in this patient population. METHODS: Eighty-one repetitive levosimendan infusions administered to 20 patients with DCM at severe or end stage of the disease in the pediatric intensive care unit were analyzed retrospectively...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#19
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27999550/the-use-of-berlin-heart-excor-vad-in-children-less-than-10-kg-a-single-center-experience
#20
Arianna Di Molfetta, Fabrizio Gandolfo, Sergio Filippelli, Gianluigi Perri, Luca Di Chiara, Roberta Iacobelli, Rachele Adorisio, Isabella Favia, Alessandra Rizza, Giuseppina Testa, Matteo Di Nardo, Antonio Amodeo
Objective: Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg...
2016: Frontiers in Physiology
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